This antibody at high titer is a serologic hallmark of MCTD but may be present in low titers in other diseases.
What is RNP?
Renal disease in MCTD is ______ (more/less/as) common than in lupus.
What is "less"?
This neurologic diagnosis is relatively unique to Sjogren's Syndrome
What is a purely sensory neuropathy?
Common rashes at onset of MCTD are: _______
What is rashes of cSLE or JDM?
This type of white blood cell is increased in tissues affected by IgG4-Related Disease.
What is the eosinophil?
(also plasma cells)
This antibody (or antibodies) is/are associated with neonatal lupus.
What are SSA (anti-Ro) and SSB (anti-La)?
The most common clinical manifestation of MCTD at presentation is ...
What is arthritis?
These proteins are associated with poor prognosis in Sjogren's Syndrome.
What are cryoglobulins?
When children with MCTD have arthritis, it is associated with which protein.
What is rheumatoid factor (RF) seropositivity?
This is the most common clinical manifestation of IgG4-related disease in children.
What is orbital disease? (44%)
Other common clinical manifestations include:
- Pancreatitis/autoimmune pancreatitis type 1 (12%)
- Cholangitis (8%)
- Pulmonary disease (8%)
This antibody is associated with disease persistence in MCTD
What is RF (rheumatoid factor)?
This rheumatic disease is associated with a 44 fold increased risk of developing lymphoma.
What is Sjogren's Syndrome?
This is the type of lymphoma that is most common for patients with Sjogren's.
What is non-Hodgkin lymphoma?
Or what is extranodal B-cell lymphoma of the MALT cell type?
This is the major source of morbidity and mortality among adults with MCTD.
What is pulmonary disease?
Pulmonary hypertension is the most frequent serious complication
This is the term for patients with some features of CTD who lack adequate clinical or diagnostic features to fit a recognizable clinical syndrome.
What is undifferentiated connective tissue disease?
Not to be confused with overlap syndrome: patients with two or more distinctly recognizable rheumatic diseases.
According to the Sharp criteria, this antibody CANNOT be present for a diagnosis of MCTD.
What is anti-Smith (Sm)?
_____ should be on your differential for a patient with retro-orbital pseudotumor.
What is IgG4 related diseases?
Name one (or more!- up to 4) clinical features thought to be predictive of lymphoma development in Sjogren's patients.
What is: persistent enlargement of parotid glands, lymphadenopathy, splenomegaly and palpable purpura?
This HLA class II specificity is associated with MCTD.
What is HLA-DR4?
Name 1 (or more! - up to 3) lab features that are associated with a predisposition to lymphoma in patients with Sjogren Syndrome.
What is mixed monoclonal cryoglobulinemia, low level of C4, monoclonal bands in serum or urine?
IgG4-Related Disease is characterized by serum levels of IgG greater than ____
What is 140 mg/dL?
Neuromyelitis optica spectrum disorder is associated with this rheumatologic diagnosis
What is Sjogren's Syndrome?
Patients with Sjogren Syndrome treated with belimumab (Benlysta) reported improvement in this symptom.
What is dryness?
No improvement in pain or fatigue
This hematologic abnormality when it occurs can be resistant to conventional therapy.
What is thrombocytopenia?
This cytokine is thought to contribute to the pathogenesis of Sjogren Syndrome.
What is IFNalpha?
IFN signature detected in PBMC of SS patients and correlated with anti-SSA and anti-SSB levels. There is increased expression of IFN-regulated genes in the salivary glands of SS patients.